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We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. Neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. Intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.
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PMID:Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl. 1070 32

Stroke mortality represents the third leading cause of death worldwide, after coronary artery disease and cancer. It has been demonstrated that in Mongolian gerbils, a unilateral hemispheric cerebral infarction can be produced following unilateral occlusion of the carotid artery because of the absence of connecting arteries between the basilar and carotid systems in these animals. The objective of this study was to comprehensively characterize the model of cerebral infarction in gerbil, clinically, biochemically and especially morphologically for prospective use in testing new therapeutic agents. Cerebral infarction was produced by ligation of the left common carotid artery in experimental gerbils. The control animals were sham-operated. One hour after surgery, 0.5 ml of 1% trypan blue was administered intraperitoneally to all animals. Initial clinical evaluations were made 8 h after surgery and every day thereafter for 30 days. On each of days 10 and 30, 4 animals were sacrificed. The degree of cerebral infarction was evaluated on the basis of clinical response, electrolyte and enzyme changes, vascular permeability of blood-brain barrier and morphological alterations. The total post-infarction mortality rate was 50%. The clinical symptoms presented as ipsilateral hemiparesis, ptosis of the eyelid, circling behavior, decreased breathing rate, decreased blood pressure and increased heart rate. Such symptoms developed within 8 h of ligation and persisted to sacrifice at day 30. Creatine kinase increased significantly on the 10th day and remained high to day 30. Increased potassium from the damaged cells and breakdown of the blood-brain barrier were first detected 72 h post-infarction. The morphological data showed evidence of brain cell necrosis, autolysis and phagocytosis 10 and 30 days post-ligation in left hemispheres. Minor intercellular edema and some cell shrinkage was evident in the right brain. Areas of focal necrosis in the vicinity of blood vessels, especially in the left brain suggested a reperfusion injury as a consequence of minimal collateral reflow from the right brain into the left brain microvasculature. Experimental infarction in gerbil recreates the ischemic conditions causing stroke in humans. The animal model may be used for evaluating the efficacy of therapeutic agents that may ameliorate the condition in man.
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PMID:Mongolian gerbil (Meriones unguiculatus) as a model of cerebral infarction for testing new therapeutic agents. 1093 31

Paracoccidioidomycosis (PCM) is a primary pulmonary infection that often disseminates to other organs and systems. Involvement of the central nervous system (CNS) is rare and due to the fact that both clinical alertness and establishment of the diagnosis are delayed, the disease progresses causing serious problems. We report here a case of neuroparacoccidioidomycosis (NPCM), observed in a 55 year-old male, who consulted due to neurological symptoms (left hemiparesis, paresthesias, right palpebral ptosis, headache, vomiting and tonic clonic seizures) of a month duration. Upon physical examination, an ulcerated granulomatous lesion was observed in the abdomen. To confirm the diagnosis a stereotactic biopsy was taken; additionally, mycological tests from the ulcerated lesion and a bronchoalveolar lavage were performed. In the latter specimens, P. brasiliensis yeast cells were visualized and later on, the brain biopsy revealed the presence of the fungus. Treatment with itraconazole (ITZ) was initiated but clinical improvement was unremarkable; due to the fact that the patient was taking sodium valproate for seizure control, drug interactions were suspected and confirmed by absence of ITZ plasma levels. The latter medication was changed to clonazepam and after several weeks, clinical improvement began to be noticed and was accompanied by diminishing P. brasiliensis antigen and antibody titers. In the PCM endemic areas, CNS involvement should be considered more often and the efficacy of itraconazole therapy should also be taken into consideration.
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PMID:Central nervous system paracoccidioidomycosis. Report of a case successfully treated with itraconazol. 1096 87

The objective of this prospective study was to investigate further the clinical features of patients with giant cell (temporal) arteritis (GCA). All patients diagnosed from July 1999 to March 2001 at the Department of Neurology of the Second Xiangya Hospital in China were included. The final diagnosis was based on clinical manifestations, a temporal artery biopsy, response to steroid, and follow-up. The American College of Rheumatology (ACR) criteria for the classification of GCA were tested in the patients identified. Sixteen patients with GCA were identified; 13 (81.25%) patients fulfilled the 1990 ACR criteria for the classification of GCA. Clinical findings included the following: mean age at disease onset 43.13 years (range 28-60) and 81.25% of the patients under the age of 50 when the disease began; men 93.75%; the common initial symptoms including new headache 62.50% and.visual symptoms 18.75%; the common clinical findings at presentation including new headache 93.75%, temporal artery abnormality 81.25%, visual abnormality 56.25%, and fever 25.00%; raised erythrocyte sedimentation rate (ESR) 68.75%; and uncommon findings including jaw claudication, ptosis, fatigue, syncope, hemiparesis; all 16 patients underwent a temporal artery biopsy; inflammatory cell infiltration 68.75% in arterial wall, fragmented internal elastica 100.00%, fibrinoid necrosis 18.75%, smooth muscle cell changes 62.50%, and thrombosis in the lumen 31.25%. The mean time from symptom onset to suspicion of GCA or biopsy was 5.52 months (range 0.25-24.33); the initial diagnosis was wrong in 87.50% of patients. These examples are too small a number to permit definite conclusion. But the results suggest that GCA may not be a rare disorder in China, mean age at disease onset was relatively young, males may be more susceptible, the clinical features of GCA have not been widely appreciated yet, there was a delay between diagnosis and treatment, and initial diagnosis was wrong in many patients.
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PMID:Giant cell arteritis in China: a prospective investigation. 1214 52

The male proband reported here was born with appropriate anthropometric parameters at term as the second child of healthy nonconsanguineous parents. His only clinical symptom was bilateral congenital cataracts with strabismus at birth, and both lenses were removed surgically at the age of 8 months. The perinatal and infantile period thereafter was clinically uneventful and his psychomotor development appeared almost normal. At the age of 6 years he was hospitalized for slight muscle weakness, minor ptosis, nystagmus and decreased physical activity. Soon after, his general condition worsened, gait ataxia presented, dysphagia and difficulty of speech followed by rapidly progressive generalized ataxia, and myopathy developed. Typical progressive gray matter degeneration with focal necrosis in the basal ganglia characteristic of the Leigh type of neuropathology could be detected by cranial MRI, the muscle histology showed ragged-red fibers. At the age of 7.5 years, unexpected left side hemiparesis with speech disability resembling that seen in MELAS syndrome developed, from which he recovered within 1.5 days. The mtDNA of the patient showed single 6.7 kb large-scale deletion harboring between 7817 and 14 536 bp. This case represents the first report of a verified mtDNA mutation associated with congenital cataracts as the first clinical sign of a later developing progressive neuromuscular disease presented with a combination of Leigh neuropathology, ragged-red fiber histopathology and stroke-like attack.
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PMID:Congenital cataract as the first symptom of a neuromuscular disease caused by a novel single large-scale mitochondrial DNA deletion. 1273 42

Garcin syndrome is characterized by an unilateral cranial nerves involvement without sensory or motor long-tract disturbances. It is usually caused by tumor infiltrating in the skull base with osteolytic changes on radiological study. We report a case of 64-year-old man with history of alcohol overintake, who admitted local hospital, because of right periorbital edema and facial swelling. He noted right ptosis 2 weeks prior to admission. Neurological examination revealed right multiple cranial nerves involvement including II, III, IV, V, and VI cranial nerves. MR imaging of the brain showed marked paranasal sinusitis and abnormal infiltration of right orbital fat. Orbital apex syndrome related to paranasal sinusitis was diagnosed, and antibiotics was administered. But a few days after admission, he developed a right VII, IX, X cranial nerve palsy. He was transferred to our hospital because of acute development of left hemiparesis and deteriorated consciousness. MR imaging of the brain showed right internal carotid artery (ICA) occlusion, and infarction in right middle cerebral artery (MCA)'s territory. The diagnostic biopsy of the paranasal sinus showed mucorales hyphae, indicating that the pathological diagnosis was mucormycosis. Despite of antibiotic therapy included of amphotericin-B administration and strict control of diabetic mellitus, his sinusitis was gradually spread. His condition progressively deteriorated, and finally died of sepsis. Post-mortem examination revealed a widespread mucor infiltration in the dura mater without skull bone invasion. This case presented with unilateral multiple cranial nerve involvements (Garcin syndrome) followed by left hemiparesis associated with rhinocerebral mucormycosis. It is suggested that mucormycosis should be considered in case of Garcin syndrome without osteolysis in the skull base.
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PMID:[Garcin syndrome in a patient with rhinocerebral mucormycosis]. 1511 47

A 29-year-old man presented with lethargy, headache, high fever, and visual disturbance. Neurological examination showed mydriatic pupil, ptosis, diminished light reflex, and ophthalmoplegia on the left. Magnetic resonance (MR) imaging showed the typical findings of pituitary apoplexy, and cerebral angiography disclosed mild narrowing of the A1 segment of the left anterior cerebral artery (ACA). Transsphenoidal tumor resection was performed. Transient severe right hemiparesis occurred directly after the operation. Computed tomography demonstrated cerebral infarction in the territory of the left Heubner's and medial lenticulostriate arteries. Pituitary apoplexy followed by cerebral infarction is very rare. Vasospasm of the perforating arteries of the ACA probably caused the cerebral infarction. Subarachnoid blood or vasoactive agents released from the tumor were the most likely cause of the vasospasm. MR imaging findings of contrast enhancement around the vessels may indicate reactive processes around the vessels.
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PMID:Cerebral infarction following pituitary apoplexy--case report. 1560 Feb 83

Strokes identified in older children typically present with sudden hemiparesis, frequent association with seizures, and occasional accompaniment of hemisensory signs or visual field defects. In this case of a left cerebral peduncle infarction, initially the patient was not found with evident right-side hemiparesis but with right-side paresthesia and mild worsening of her underlying left-side weakness, including weakness of left facial expression and left ptosis. Her right limbs became gradually weaker in the first 2 days. These bizarre clinical presentations, the mild worsening of the underlying left-side weakness, and the delayed presentation of right-side hemiparesis added to the difficulty of making a diagnosis and of the initial localization. In addition, the magnetic resonance angiography incidentally found an abnormal vessel, which was additional and serpiginous to the right posterior fossa. The right internal carotid artery was markedly smaller than the left one, and a defect was seen around the area of the presumed distal right internal carotid artery. The abnormal cerebral vasculature might be the cause of the unique clinical presentation and might be the contributing factor to the recurrence of her stroke. In conclusion, the authors report a special case of recurrent stroke on the basis of aberrant cerebral vasculature.
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PMID:Acute midbrain infarction in a child with intracranial carotid artery hypoplasia and aberrant cerebral vasculature: a case report. 1762 31

We report on a four-year-old girl with head trauma caused by a motor vehicle accident. She presented with delirium, oculomotor palsy and ptosis in her left eye, left hemiparesis, and pyramidal signs in all extremities. Computed tomography on the day of admission showed diffuse cerebral edema with right-sided predominance. Magnetic resonance images on day 3 of admission showed lesions of diffuse axonal injury and contusion in the corpus callosum and right occipital and bilateral temporal lobes. There was a low-intensity lesion in the white matter of the right hemisphere on T2-weighted images, fluid-attenuated inversion recovery, T2()-weighted images, apparent diffusion coefficient maps and diffusion-weighted images. This low-intensity lesion disappeared by day 7, and a transient brain atrophy in the right hemisphere appeared on day 28. The low signal intensity in the cerebral white matter was apparently different from that associated with contusion and typical diffuse axonal injury, and might represent a late-onset accumulation of non-heme iron and free radicals in the white matter after head trauma.
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PMID:Low signal intensity and increased anisotropy on magnetic resonance imaging in the white matter lesion after head trauma: unrecognized findings of diffuse axonal injury. 1766 30

Previous reports suggest that right hemispheric lesions may produce a variety of disorders in eye opening, including bilateral eyelid ptosis. More recently, bilateral ptosis and a severe deficit of upward gaze while preserving the downward gaze have been reported in patients with right hemispheric infarction. In the present report, we describe 2 patients who showed downward gaze palsy in addition to upward gaze palsy, accompanied by bilateral ptosis following right putaminal hemorrhage. The 2 patients displayed acute onset of similar ocular manifestations, such as difficulty in eye opening, upward and downward gaze palsy, and conjugate horizontal deviation in addition to left hemiparesis. The principal cause of difficulty in eye opening was considered to be bilateral ptosis. Computed tomography performed on admission revealed a moderate-sized hematoma in the right putamen. There was no evidence of midbrain involvement. These ocular symptoms were restored within a few weeks after the onset of stroke. The ocular manifestations noted in our patients suggest that the descending pathways arising from the supranuclear centers to the midbrain, which control vertical movement of the eyes and the eyelids, may be impaired by putaminal hemorrhage. To our knowledge, this is the first report of such ocular manifestations in the right hemispheric lesion.
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PMID:Vertical gaze palsy associated with bilateral ptosis following right putaminal hemorrhage. 1790 65


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